Abstract:
Ceramide (Cer) plays an essential role in photoreceptor cell death in the retina. On the one hand, Cer accumulation emerges as a common feature during retina neurodegeneration, leading to the death of photoreceptors. On the other hand, Cer deficiency has also recently been associated with retinal dysfunction and degeneration. Although more and more evidence supports the importance of maintaining Cer homeostasis in the retina, mechanistic explanations of the observed phenotypes, especially in the context of Cer deficiency, are still lacking. An enhanced understanding of Cer\'s role in the retina will help us explore the underlying molecular basis for clinical phenotypes of retinal dystrophies and provide us with potential therapeutic targets.
摘要:
神经酰胺(Cer)在视网膜感光细胞死亡中起重要作用。一方面,在视网膜神经变性过程中,Cer积累是一个共同的特征,导致光感受器死亡。另一方面,Cer缺乏最近也与视网膜功能障碍和变性有关。尽管越来越多的证据支持维持视网膜Cer稳态的重要性,对观察到的表型的机械解释,尤其是在Cer缺乏的情况下,仍然缺乏。增强对Cer在视网膜中的作用的理解将有助于我们探索视网膜营养不良临床表型的潜在分子基础,并为我们提供潜在的治疗靶点。